What is the recommended treatment and management approach for a patient suspected of having Guillain-Barré Syndrome (GBS)?

Guillain-Barré Syndrome: Treatment and Management

First-Line Treatment

Initiate intravenous immunoglobulin (IVIg) at 0.4 g/kg/day for 5 consecutive days (total dose 2 g/kg) immediately in patients unable to walk unaided or showing rapid progression, respiratory compromise, dysphagia, facial weakness, or bulbar weakness. 1, 2

• IVIg is preferred over plasma exchange as first-line therapy because it is easier to administer, more widely available, has higher completion rates, and requires fewer monitoring considerations 1
• Plasma exchange (12-15 L over 4-5 sessions in 1-2 weeks) is equally effective and should be considered as an alternative if IVIg is unavailable or contraindicated 2
• Do not use corticosteroids alone - randomized controlled trials show no benefit and oral corticosteroids may worsen outcomes 1, 2
• Treatment should be initiated as early as possible, ideally within 2 weeks of symptom onset for IVIg and within 4 weeks for plasma exchange 2

Critical Respiratory Monitoring

Admit all patients to a unit with rapid ICU transfer capability and monitor respiratory function using the "20/30/40 rule" to predict impending respiratory failure. 3, 1

• Patient is at high risk of respiratory failure if:
  • Vital capacity <20 ml/kg, OR 3, 1
  • Maximum inspiratory pressure <30 cmH₂O, OR 3, 1
  • Maximum expiratory pressure <40 cmH₂O 3, 1
• Single breath count ≤19 predicts requirement for mechanical ventilation 3
• Up to 30% of patients develop respiratory failure requiring mechanical ventilation 4
• Monitor vital capacity, maximum inspiratory/expiratory pressures, and use of accessory respiratory muscles frequently 1

Neurological and Autonomic Monitoring

Perform frequent assessments of motor strength using the Medical Research Council scale, functional disability using the GBS disability scale, and continuous monitoring for autonomic dysfunction. 3

• Monitor for swallowing and coughing difficulties - assess safe swallowing and provide nutritional support if dysphagia present 3, 1
• Perform electrocardiography and monitor heart rate, blood pressure, and bowel/bladder function for autonomic dysfunction 3
• Two-thirds of deaths occur during the recovery phase from cardiovascular and respiratory complications - maintain vigilance even after apparent improvement 3, 5
• Stay especially alert in patients recently transferred from ICU and those with cardiovascular risk factors 3

Diagnostic Confirmation

Obtain CSF analysis showing elevated protein with normal cell count (albumino-cytological dissociation), though normal protein in the first week does not exclude GBS. 3

• CSF protein is normal in 30-50% of patients in the first week and 10-30% in the second week 3
• Marked pleocytosis (>50 cells/μl) suggests alternative diagnoses such as leptomeningeal malignancy or infectious polyradiculitis 3
• Electrodiagnostic studies support the diagnosis but are not required to initiate treatment 3
• Anti-GQ1b antibody testing should be performed if Miller Fisher syndrome is suspected (found in up to 90% of MFS cases) 3
• Do not delay treatment while waiting for antibody test results 3

Multidisciplinary Supportive Care

Implement comprehensive complication prevention including DVT prophylaxis, pressure ulcer prevention, pain management, and psychological support from a multidisciplinary team. 3, 1

• Manage neuropathic pain with gabapentin, pregabalin, or duloxetine - avoid opioids 1, 2
• Prevent pressure ulcers, hospital-acquired infections (pneumonia, UTI), and deep vein thrombosis using standard prophylactic measures 3, 1
• Address corneal ulceration in patients with facial palsy and limb contractures in patients with severe weakness 3
• Specifically ask about pain, hallucinations, anxiety, and depression - these symptoms are frequent but often underrecognized, especially in ICU patients with limited communication 3
• Remember that patients with complete paralysis usually have intact consciousness, vision, and hearing - explain procedures and be mindful of bedside conversations 3

Medications to Avoid

Do not administer β-blockers, IV magnesium, fluoroquinolones, aminoglycosides, or macrolides as these worsen neuromuscular function. 1, 5

Management of Insufficient Response

Approximately 40% of patients do not improve in the first 4 weeks following treatment - this does not necessarily indicate treatment failure. 3, 6

• Treatment-related fluctuations (TRFs) occur in 6-10% of patients within 2 months after initial improvement 3, 6
• Repeat the full course of IVIg or plasma exchange for TRFs 6, 7
• Suspect chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) if three or more TRFs occur or clinical deterioration continues ≥8 weeks after onset - this occurs in approximately 5% of initially diagnosed GBS patients 6, 2
• Evidence for second-course IVIg in poor-prognosis patients without TRFs is limited and not currently recommended 2

Special Populations

• Children: Use the same 5-day IVIg regimen (0.4 g/kg/day) rather than accelerated 2-day protocols due to higher TRF rates with shorter regimens 1
• Pregnant women: IVIg is preferred over plasma exchange due to fewer monitoring requirements, though neither is contraindicated 1
• Miller Fisher Syndrome: Treatment is generally not recommended as most recover completely within 6 months without intervention, though close monitoring is essential 1
• Immune checkpoint inhibitor-related GBS: Discontinue the causative agent permanently and consider concurrent corticosteroids with IVIg or plasma exchange 1

Prognostic Assessment

Use the modified Erasmus GBS outcome score (mEGOS) to calculate individual probability of regaining walking ability and the modified EGRIS to assess ventilation risk. 6, 2

• Approximately 80% of patients regain independent walking ability by 6 months 1, 5
• Mortality is 3-10%, primarily from cardiovascular and respiratory complications 1, 5
• Risk factors for poor outcome include advanced age and severe disease at onset 6, 5
• About 20% of patients remain unable to walk after 6 months, and many have persistent pain, fatigue, or other residual complaints 7